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LIBRIS Formathandbok  (Information om MARC21)
FältnamnIndikatorerMetadata
00004980naa a2200529 4500
001oai:DiVA.org:umu-194848
003SwePub
008220607s2022 | |||||||||||000 ||eng|
024a https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-1948482 URI
024a https://doi.org/10.1080/13506129.2022.20432702 DOI
040 a (SwePub)umu
041 a engb eng
042 9 SwePub
072 7a ref2 swepub-contenttype
072 7a art2 swepub-publicationtype
100a Barroso, Fabio A.u Institute for Neurological Research, FLENI, Buenos Aires, Argentina4 aut
2451 0a Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS)
264 c 2022-04-22
264 1b Taylor & Francis,c 2022
338 a electronic2 rdacarrier
520 a Background: Autonomic dysfunction is common in transthyretin amyloidosis (ATTR amyloidosis), but its frequency, characteristics, and quality-of-life (QoL) impact are not well understood.Methods: The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing, global, longitudinal survey of patients with ATTR amyloidosis, including patients with inherited (ATTRv) and wild-type (ATTRwt) disease and asymptomatic patients with TTR mutations (ClinicalTrials.gov: NCT00628745). In a descriptive analysis, characteristics and Norfolk QoL-DN total (TQoL) scores at enrolment were compared in patients with vs without autonomic dysfunction (analysis cut-off: 1 August 2020).Results: Autonomic dysfunction occurred in 1181/2922 (40.4%) symptomatic patients, and more commonly in ATTRv (1107/1181 [93.7%]) than ATTRwt (74/1181 [6.3%]) amyloidosis. Time (mean [SD]) from ATTR amyloidosis symptom onset to first autonomic dysfunction symptom was shorter in ATTRv (3.4 [5.7] years) than ATTRwt disease (9.7 [10.4]). In ATTRv disease, patients with vs without autonomic dysfunction had worse QoL (TQoL, 47.3 [33.2] vs 16.1 [18.1]); in ATTRwt disease, those with vs without autonomic dysfunction had similar QoL (23.0 [18.2] vs 19.9 [20.5]).Conclusions: Autonomic dysfunction was more common and presented earlier in symptomatic ATTRv than ATTRwt amyloidosis and adversely affected QoL in ATTRv disease. These THAOS findings may aid clinicians in diagnosing and treating patients with ATTR amyloidosis.Trial registration: ClinicalTrials.gov: NCT00628745.
650 7a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Neurologi0 (SwePub)302072 hsv//swe
650 7a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Neurology0 (SwePub)302072 hsv//eng
653 a Amyloidosis
653 a autonomic nervous system
653 a neuropathy
653 a registry
653 a transthyretin
700a Coelho, Teresau Unidade Corino Andrade, Hospital Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal4 aut
700a Dispenzieri, Angelau Division of Hematology, Mayo Clinic, MN, Rochester, United States4 aut
700a Conceição, Isabelu Hospital de Santa Maria–CHULN, FML Universidade de Lisboa, Lisbon, Portugal4 aut
700a Waddington-Cruz, Marciau CEPARM, University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil4 aut
700a Wixner, Jonasu Umeå universitet,Avdelningen för medicin4 aut0 (Swepub:umu)jowi0065
700a Maurer, Mathew S.u Columbia University College of Physicians and Surgeons, NY, New York, United States4 aut
700a Rapezzi, Claudiou Cardiological Center, University of Ferrara, Ferrara, Italy; Maria Cecilia Hospital, GVM Care & Research, Cotignola (RA), Italy4 aut
700a Planté-Bordeneuve, Violaineu Department of Neurology, East-Paris University, Hospital Henri Mondor, Assistance Publique Hopitaux de Paris, INSERM U955 Team 10 “Biology of the Neuro-Muscular System”, Crétei, France4 aut
700a Kristen, Arnt V.u Department of Cardiology, Angiology, and Respiratory Medicine, Medical University of Heidelberg, Heidelberg, Germany4 aut
700a González-Duarte, Alejandrau Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico4 aut
700a Chapman, Dougu Pfizer Inc, NY, New York, United States4 aut
700a Stewart, Michelleu Pfizer Inc, CT, Groton, United States4 aut
700a Amass, Leslieu Pfizer Inc, CT, Groton, United States4 aut
710a Institute for Neurological Research, FLENI, Buenos Aires, Argentinab Unidade Corino Andrade, Hospital Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal4 org
773t Amyloidd : Taylor & Francisg 29:3, s. 175-183q 29:3<175-183x 1350-6129x 1744-2818
856u https://doi.org/10.1080/13506129.2022.2043270y Fulltext
856u https://umu.diva-portal.org/smash/get/diva2:1665360/FULLTEXT02.pdfx primaryx Raw objecty fulltext:print
8564 8u https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-194848
8564 8u https://doi.org/10.1080/13506129.2022.2043270

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